Related Experiment Videos
Pulmonary arteriovenous shunting in children with liver disease
Insights
Pulmonary arteriovenous shunting (PAVS) in children with cirrhosis can cause hypoxemia and may resolve after liver transplantation. Early screening and transplantation are crucial for improving outcomes in PAVS patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Cardiopulmonary Medicine
Background:
- Pulmonary arteriovenous shunting (PAVS) is a serious complication of cirrhosis, potentially leading to hypoxemia.
- PAVS may regress following liver transplantation.
- The incidence and characteristics of PAVS in pediatric cirrhosis require further investigation.
Purpose of the Study:
- To investigate the occurrence, clinical presentation, and outcomes of PAVS in children with cirrhosis.
- To evaluate the impact of liver transplantation on PAVS regression.
- To identify predictors of prognosis in pediatric PAVS.
Main Methods:
- Retrospective analysis of 26 children diagnosed with PAVS and cirrhosis or portal vein obstruction.
- Diagnostic methods included technetium Tc 99m microaggregated albumin pulmonary scanning and assessment of alveoloarterial oxygen gradients.
- Clinical data, including oxygenation levels (PaO2), cardiac index, and treatment outcomes, were analyzed.
Main Results:
- PAVS was observed in children with cirrhosis and portal vein obstruction, presenting with cyanosis or dyspnea.
- Children with biliary atresia and polysplenia syndrome showed earlier onset and more severe shunting.
- Liver transplantation led to PAVS regression in survivors, with higher PaO2 on 100% oxygen predicting better outcomes.
Conclusions:
- PAVS is a significant complication in pediatric portal hypertension, particularly in biliary atresia and polysplenia syndrome.
- Early liver transplantation is effective in reversing PAVS.
- Systematic screening for PAVS is recommended in at-risk pediatric populations.
Abstract:
Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin pulmonary scanning or a high alveoloarterial O2 gradient or both. Cyanosis or dyspnea or both occurred at ages ranging from 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome (p < 0.01). Mean arterial oxygen tension (PaO2) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O2. Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome (p < 0.01). Seven untreated children died 3 months to 8 years after the diagnosis of PAVS. Eleven underwent liver transplantation: seven are alive (follow-up, 1 to 4 years) and have no signs of PAVS. The PaO2 value during breathing of 100% O2 was > 300 mm Hg in the survivors and < 200 mm Hg in the four nonsurvivors (p < 0.01). These results indicate (1) that PAVS can occur at any age in children with portal hypertension, and that the risk is highest and earliest in children with biliary atresia and polysplenia syndrome, (2) that early liver transplantation allows regression of PAVS, and (3) that the prognosis may in part be related to the level of PaO2 while the patient is breathing 100% O2. The results indicate that systematic screening for PAVS should be part of the examination of these children.